path neuro formatted Flashcards
With regard to CC dysgenesis, which one is false
1. genu always present in partial
2. cingulate gyrus normal
3. medial parietal sulci affected
4. foramen monro enlarged
5. assoc with AC II
. cingulate gyrus normal - F - dysgenesis of cingulate gyrus / persistent eversion of cingulate gyrus
9) CC dysgenesis, false (TW)
- genu always present in partial - T - depending on time of arrested growth (anteroposterior development of genu, body, splenium, however, rostrum forming last). Can have: genu only, genu + part of body, genu + entire body, genu + body + splenium (without rostrum).
- cingulate gyrus normal - F - dysgenesis of cingulate gyrus / persistent eversion of cingulate gyrus
- medial parietal sulci affected - T - radial array pattern of medial cerebral sulci, gyri “point to” 3rd ventricle
- foramen monro enlarged - T - enlarged / elongated foramina of Munro
- assoc with AC II - T - 7%. Also assoc with DW cyst, interhemispheric arachnoid cyst, hydrocephalus, lipoma of CC, porencephaly, holoprosencephaly, polymicrogyria. CC segments (front to back): Lamina rostralis (unmyelinated), Rostrum, Genu, Body / isthmus, Splenium (myelinated).
*Presume AC II = Arnold-Chiari type II malformation
- 28 yo F 6/52 post partum, hypopituitarism, most likely:
- Empty sella
- Pituitary carcinoma
- Lymphocytic infiltration
- Sheehan syndrome
Answer: Lymphocytic infiltration or Sheehan syndrome (most likely Lymphocytic infiltration as no other history given).
ROBBINS
Causes of hypopituitarism
- Tumors
- TBI and SAH
- Radiation/Surgery
- Pituitary apoplexy: sudden hemorrhage into pituitary gland, often occurring into a pituitary adenoma
- Ischemic Necrosis of Pituitary (Sheehan syndrome)
- Rathke Cleft cyst
- Empty sella syndrome
EMPTY SELLA SYNDROM
- primary: defect in diaphragma sella allows arachnoid mater and CSF to herniate and compress the pituitary. Classically occurs in multiparous obese women.
- Secondary: Pituitary adenoma enlarges sella, then surgical removed or undergoes infarction.
LYMPHOCYTIC HYPOPHYSITIS
- Autoimmune, inflammatory disorder
- Peripartum women, with headache, multiple endocrine deficiencies.
SHEEHAN SYNDROME
- Postpartum necrosis of the anterior pituitary
- during pregnancy, anterior pituitary enlarges 2x its normal size
- no accompanying increase in blood supply, so relative hypoxia.
- obstetric hemorrhage/shock precipitates infarction of anterior lobe as a result.
- 73.APRIL02 A 34 year old, mildly retarded woman is having a CT scan for presumed meningioma. She has some small nodules on her face, coloured nodules on her iris, and brown macules 2 – 3 cm in size on her hands and neck. Her parents, who accompany her, are both normal. The most likely diagnosis is
- Type I Neurofibromatosis
- Type II Neurofibromatosis
- Turcot’s syndrome
- Tuberous Sclerosis
- Trisomy 21
- 73.APRIL02 A 34 year old, mildly retarded woman is having a CT scan for presumed meningioma. She has some small nodules on her face, coloured nodules on her iris, and brown macules 2 – 3 cm in size on her hands and neck. Her parents, who accompany her, are both normal. The most likely diagnosis is Answer:
- Type I Neurofibromatosis
- Type I Neurofibromatosis
- Type II Neurofibromatosis
- Turcot’s syndrome
- Tuberous Sclerosis
- Trisomy 21
o 50% spontaneous / 50% AD
o Cutaneous Hyperpigmented Macules (Café au Lait) o Pigmented Nodules Of The Iris (Lisch Nodules)
- Commonest sites for ependymoma, which is least likely:
- Periventricular areas
- Lateral and third ventricle in infants
- Fourth ventricle in children
- Spine in adults
ANSWER:2. Lateral and third ventricle in infants - anaplastic ependymoma (rare), usually in infants and children. See below
- Commonest sites for ependymoma, which is least likely: (GC)
.1. Periventricular areas T - supratentorial ependymoma is more commonly seated in the brain parenchyma, typically arising near the trigone of the lateral ventricle. Thought to arise from embryonic rests of ependymal tissue trapped in the developing cerebral hemispheres. Tend to be larger in size, more often cystic components cf. infratentorial. [RG 2005, eMedicine]
- Lateral and third ventricle in infants - anaplastic ependymoma (rare), usually in infants and children. See below.
- Fourth ventricle in children T - most commonly arises from floor of 4th ventricle.
- Spine in adults T - common site, better able to excise completely and generally better prognosis. Most common intramedullary spinal neoplasm in adults.
- In HSV I encephalitis, which is least correct
- Age 50-70 years
- Typically involves superior frontal lobes
- Commonest presentation is headache
- May present with seizures, ataxia and lethargy
Answer: Typically involves superior frontal lobes (least correct)
FROM ROBBINS
- occurs most commonly in children and young adults (although STATDX says bimodal with 50% over 50 years old, but can occur at any age).
- Encephalitis starts in and most severely involves the inferior and medial regions of the temporal lobes, and the orbital gyrus of the frontal lobes.
- Typical presentation: alterations in mood, memory, and behaviour. (From statdx: fever, headaches, seizures, viral prodrome).
- In some individuals, follows a subacute course (weakness, lethargy, ataxia, seizures) over 4-6 weeks.
- 74.APRIL02 A 42-year-old alcoholic presents with confusion, nystagmus, opthalmoplegia ataxic gait but normal speech and facial movements. An MRI is ordered, These findings would be best be explained by;
- Cerebellar infarct
- Marchiafava-Bignami syndrome
- Central pontine myelinolysis
- Wernicke syndrome
- Korsakoff syndrome
- Wernicke syndrome
- A new Nuc Med agent can attach to the amyloid in neuritic plaques. Which is most correct?
- Should help exclude congophilic angiopathy in the elderly
- Uptake would allow distinction between Alzheimers and age matched Parkinsons disease
- Alzheimers patients should have greater uptake in the medial temporal lobe than age matched control patients
- Cerebellar uptake would suggest ataxic telangiectasia
- Deep cerebral uptake would suggest multi-infarct dementia or MS
ANSWER:3. Alzheimers patients should have greater uptake in the medial temporal lobe than age matched control patients - T - degenerative process starts in medial temporal lobe, spreads to parahippocampal gyrus, temporal and frontal lobes, and finally involves motor and visual cortex. Get parietal and temporal cortical atrophy with disproportionate hippocampal volume loss. Predominates ijn medial temporal and pareital lobes.
- A new Nuc Med agent can attach to the amyloid in neuritic plaques. Which is most correct? (TW)
- Should help exclude congophilic angiopathy in the elderly - F - amyloid deposition not specific from amyloid angiopathy
- Uptake would allow distinction between Alzheimers and age matched Parkinsons disease - Alzheimers neuritic plaques have amyloid core. Plaques can be found in non-demented patients but in lower numbers.
- Alzheimers patients should have greater uptake in the medial temporal lobe than age matched control patients - T - degenerative process starts in medial temporal lobe, spreads to parahippocampal gyrus, temporal and frontal lobes, and finally involves motor and visual cortex. Get parietal and temporal cortical atrophy with disproportionate hippocampal volume loss. Predominates ijn medial temporal and pareital lobes.
- Cerebellar uptake would suggest ataxic telangiectasia - F - pathology is neuronal degredation + atrophy of cerebellar cortex (? from vascular anomalies).
- Deep cerebral uptake would suggest multi-infarct dementia or MS - F - infarcts, demyelination.
- acromegaly 3cm adrenal lesion likely a
- met
- adenocarcinoma
- incidental adenoma
LW: likely incomplete recall:
> 80% of acromegaly due to pituitary GH release.
Rare case reports of Phaeochromocytomas secreting GHRH and resulting in acromegaly. Not in Robbins.
Hence incidentaloma is favoured option, as they are relatively common, and unlikely related to acromegaly in the provided details. While a met or adenocarcinoma of adrenal gland wont / unlikely to secrete GH or GHRH to cause acromegaly
. incidental adenoma – T – most likely. Incidental adenomas found in 1-2% of population.
- acromegaly 3cm adrenal lesion likely a (TW)
- met – F – no reason to suspect mets
- adenocarcinoma – F – adrenal carcinoma rare.
- incidental adenoma – T – most likely. Incidental adenomas found in 1-2% of population. Acromegaly – excess growth hormone due to eosinophilic adenoma / hyperplasia in anterior pituitary.
- Multiple sclerosis, distribution:
a. Ovoid lesions with long axis oriented parallel to ventricular walls
b. Subcortical U fibres spared
c. 40% of spinal lesions occur without coexistent intracranial plaques
d. Perivenular inflammation
e. Predilection for thoracic cord
Perivenular inflammation T - at junction of pial veins 10.
Multiple sclerosis, distribution: (GC)
a. Ovoid lesions with long axis oriented parallel to ventricular walls F - perpendicular, aka Dawson’s fingers.
b. Subcortical U fibres spared F - not spared, 10% of MS plaques occur in grey matter.
c. 40% of spinal lesions occur without coexistent intracranial plaques F - 12-33%
d. Perivenular inflammation T - at junction of pial veins
e. Predilection for thoracic cord F - cervical cord [Dahnert]
- 75.APRIL02 A 46-year-old man with non-Hodgkin’s lymphoma suffers increasing headache and drowsiness over 2 weeks. CSF examination shows small round cells with a thick gelatinous capsule, well appreciated on Indian ink suspension. This is most likely to represent:
- Cerebral involvement with candida
- Cerebral involvement with tuberculosis
- Cerebral involvement with cryptococcus
- Cerebral involvement with non-Hodgkin’s lymphoma
- Transformation of the lymphoma and secondary cerebral involvement with acute lymphoblastic leukaemia
- Cerebral involvement with cryptococcus
• May show encapsulated yeasts of Cryptococci on India Ink or Cryptococcal antigen, or in tissue sections by PAS, mucicarmine and silver stains (esp. frequent in debilitated or immunocompromised patients)
- An unconscious alcoholic with multiple medical problems is resuscitated with IV fluids and thiamine. MRI shows white matter lesions in pons, tegmentum and deep cerebral white matter. Which is most likely?
- Central pontine myelinolysis
- Beri-beri
- Wernicke – korsakoffs
- ETOH encephalomyelitis
- Severe combined degeneration of the white matter
ANSWER: 11. Central pontine myelinolysis - T - Now referred to as Osmotic demyelination syndrome (previously CPM, or extrapontine myelinolysis). Demyelination due to (usually) osmotic stress related problems - but exact mechanism is not known. Isolated pons lesion is most common. Combined type: central and extrapontine areas (basal ganglia, cerebellar white matter, thalamus, caudate nucleus, subcortical cerebral white matter, corona radiata, lateral geniculate body).
- An unconscious alcoholic with multiple medical problems is resuscitated with IV fluids and thiamine. MRI shows white matter lesions in pons, tegmentum and deep cerebral white matter. Which is most likely? (TW)
ANSWER: 11. Central pontine myelinolysis - T - Now referred to as Osmotic demyelination syndrome (previously CPM, or extrapontine myelinolysis). Demyelination due to (usually) osmotic stress related problems - but exact mechanism is not known. Isolated pons lesion is most common. Combined type: central and extrapontine areas (basal ganglia, cerebellar white matter, thalamus, caudate nucleus, subcortical cerebral white matter, corona radiata, lateral geniculate body).
- Beri-beri - F - thiamine deficiency (B1), and often manifests in cardiovascular collapse (wet beriberi). Nervous involvement - symmetric impairment of sensory, motor, and reflex functions (dry beriberi).
- Wernicke – korsakoffs - F - Wernicke encephalopathy - mamillary body, medial thalamus, hypothalamus, PAG abnormal signal. Alcoholic encephalopathy - disproportionate superior vermain atrophy.
- ETOH encephalomyelitis - F
- Periventricular mass in patient renal transplant
- Primary lymphoma
- GBM
- Secondary lymphoma
- Primary lymphoma - T - most notable risk factor for development of primary CNS lymphoma is immunodeficiency. Solid organ transplant patients have 30-50x higher incidence of lymphoproliferative disorder cf general population.
- 23.03.89 Pick’s disease, UNCOMMON FINDING ? Dan p314
- Assymetrical atrophy
- Predominant frontal lobes
- Cortical atrophy
- Involvement of posterior 2/3 superior temporal gyrus
- Putamen changes
Answer: Involvement of the posterior 2/3 superior temporal gyrus (Uncommon finding)
FROM STATDX
Gross pathological/surgical features
- Gross atrophy of frontal &/or anterior temporal lobes
- Firm cortical gray matter (gloss) &/or basal ganglia atrophy.
- Soft retracted subcortical WM
From ROBBINS
- pronounced frequently asymmetric atrophy of the frontal and temporal lobes with conspicuous sparing of the posterior 2/3 of the superior temporal gyrus
- rare involvement of either parietal or occipital lobes
- atrophy severe, “knife-edged” appearance.
- 34/40 fetus with large mass protruding posteriorly from sacrum. No evidence of Chiari malformation. Most likely is
- Benign sacrococcygeal teratoma
- Malignant sacrococcygeal teratoma
- Congenital neuroblastoma
- Imperforate cloacal membrane
- Mature ganglioneuroma
Answer: Benign sacrococcygeal teratoma
ROBBINS
Sacrococcygeal teratomas are the most common teratomas of childhood, accounting for 40% or more of cases. Girls?boys.
- 75% are mature/benign. Encountered in infants <4 months.
- 10% are associated with congenital anomalies, primarily defects of the hindgut and cloacal region and other midline defects (meningocele, spina bifida).
- 17% are immature/malignant, and are seen mainly in older children.
Congenital neuroblastoma
- most common extracranial solid malignancy in children.
- Most commonly arises from adrenal gland but can arise anywhere along sympatheti chain - so would be anterior and typically fills the pelvis.
Mature ganglioneuroma
- arise from primitive sympathetic ganglion cells, so symp chain - and anterior mass in pelvis.
- 40 year old female with stroke, underlying cause least likely is
- Atherosclerosis
- Dissection
- Coarctation of aorta
- Giant cell arteritis
- Mitral valve prolapse
ANSWER:4. Giant cell arteritis - F - GCA is a chronic vasculitis of large and medium sized vessels. Mean age at Dx is approx 72yo, and the disease essentially never occurs in individuals younger than 50yo (UpToDate).
- 40 year old female with stroke, underlying cause least likely is (TW)
- Atherosclerosis - T
- Dissection - T
- Coarctation of aorta - T- congenital or acquired (eg inflammatory diseases of aort such as Takayasu). Previously undiagnosed adults - classic presenting sign is hypertension.
- Giant cell arteritis - F - GCA is a chronic vasculitis of large and medium sized vessels. Mean age at Dx is approx 72yo, and the disease essentially never occurs in individuals younger than 50yo (UpToDate).
- Mitral valve prolapse - T - most common congenital cause of MR in adults. Natural history of MVP is generall benign, but serious complications do occur; the most common are infective endocarditis, CVAs, need for MV surgery, death.
- 16 year old with Freidrichs ataxia has a poor quality MRI. Most likely cause is
- Intention tremor
- Recurrent facial tic
- Orthopnoea
- Salaam spasms
- Hemiballismus
- Orthopnoea
Friedrich ataxia - AR degenerative disorder. Most common hereditary ataxia. Most cases caused by loss of function in frataxin gene (frataxin is a mitochondrial protein whose prescise function is unknown).Neuropathology - degeneration of posterior columns and the spinocerebellar tracts of the spinal cord and loss of the larger sensory cells of the dorsal root ganglia.Major clinical manifestations of FQ - neurologic dysfunction, cardiomyopathy, and diabetes mellitus. Neuro - ataxia of limbs, absence of lower limb reflexes, and presence of pyramidial signs. Early loss of position and vibration sense. Cardiomyopathy - concentric LVH, asymmetric septal hypertrophy, and globally decreased LVF patterns of disease.Major causes of death are complications related to the cardiomyopathy or bulbar dysfunction, leading to an inability to protect the airway.
- 23.03.86 Mycotic aneurysms ? Rob p813
- Rarely bleed
- Peripheral arterial
- Circle of Willis
- Peripheral arterial in the brain anyway.
- Sep03.63 IV contrast, 4 -12 hrs afterwards, it results in skin necrosis. It’s a repeatable response. What type of reaction is it?
- Type 1 hypersensitivity
- Type 2
- Type 3
- Type 4
- Non-immune reaction
ANSWER: 3. Type 3 antigen antibody complex
- Sep03.63 IV contrast, 4 -12 hrs afterwards, it results in skin necrosis. It’s a repeatable response. What type of reaction is it? (TW)
- Type 1 hypersensitivity - F- Anaphylactic type. Rapidly developing immunologic reaction developing within minutes after combination of antigen with antibody bound to mast cells or basophils in individuals previously sensitized to antigen
- Type 2 - F - Cytotoxic type. Mediated by antibodies directed toward antigens present on surface of cells or other tissue component (subtypes: complement-dependent reactions; antibody-dependent cell mediated cytotoxicity; antibody mediated cellular dysfunction)
- Type 3 - T - Immunocomplex disease. Induced by antigen-antibody complexes that produce tissue damage as a result of their capacity to active the complement system. Can be generalized (immune complexes formed in circulation adn deposited in many organs etc), or localized to particular organs such as kidney (GN), joints (arthritis) or the small blood vessels of the skin if the complexes are formed an deposited locally (the local Arthus reaction). See below.
- Type 4 - F - Cell mediated (delayed). Initiated by specifically sensitized T lymphocytes.
- Non-immune reaction - F - unless someone is repeatedly extravasating a lot of contrast! Maybe fun, but not ethical.Local immune complex disease (Arthus reaction)
- type III hypersensitivity reaction. Localized area of tissue necrosis resulting from acute immune complex vasculitis, usually elicited in the skin. Reaction can be produced by intracutaneous injection of antigen in an immune patient having circulating antibodies against the antigen. Unlike IgE mediated type I reactions, which appear immediately, the Arthus lesion develops over a few hours and reaches a peak 4-10hrs after injection, when it can be seen as an area of visible oedema with severe hemorrhage followed occasionally by ulceration.
- 88.APRIL02 What are the most commonly involved organisms in cerebral abscesses?
- Gram negative anaerobes
- Streptococci and staphylococci
- Haemophilus and menigococcus
- Heemophilus and pneumococcus
- Staphylococci and mycobacteria
- Streptococci and staphylococci
- Hemorrhagic areas in cerebrum on CT. Least likely
- Recent pelvic fracture
- Past rheumatic fever
- Active mastoiditis
- Recent neck manipulation
- Recent placental abruption
ANSWER:4. Recent neck manipulation - F - vert dissection, but probably least likely option. Population-based, case-control study found pts under 45yo, those with bertebrobasilar dissection or occlusion were 5x more likley than controls to have visited a chripractor in the previous week. Actual incidence reports vary (1 per 400 000 manipulations, to 2 per million).
***LJS - agree. Although at first glance all correct, neck manipulation would cause vertebral dissection, which is more likely to cause posterior fossa haemorrhage (cerebellar vs cerebral)
- Hemorrhagic areas in cerebrum on CT. Least likely (TW & GC)
- Recent pelvic fracture - T - fat emboli syndrome most commonly associated with long bone and pelvic fractures. Need pulmonary-precapillary shunt or shunt across pulmonary capillary bed, OR via PFO (ie absence of PFO doesn’t exclude it). Fat globules < um can traverse the pulmonary microvasculature (in dogs).
- Past rheumatic fever - T - risk for valvular disease / IE - emboli (could also be anticoagulated). Mitral stenosis - thromboembolic events of which 40% involve the brain or a large pulmonary embolism.
- Active mastoiditis - T - direct extension / infection, localised abscess / encephalitis, venous sinus thrombosis + infarctions, typically haemorrhagic.
- Recent neck manipulation - F - vert dissection, but probably least likely option. Population-based, case-control study found pts under 45yo, those with bertebrobasilar dissection or occlusion were 5x more likley than controls to have visited a chripractor in the previous week. Actual incidence reports vary (1 per 400 000 manipulations, to 2 per million).
- Recent placental abruption - T - presuming this refers to the mother (‘recent placental abruption’) and not the fetus. There is a risk of abuption with eclampsia / HTN. Abruption can result in severe hypovoluemia, and coagulopathy. Severe abruption 1 in 830 deliveries - of which DIC is a common manifestation with CNS involvement in 2% (coma, delirium, mocrothrombi, haemorrhage, hypoperfusion). UpToDate.
- Patient with brain tumour that appears cystic with solid component— least likely
- Haemangioblastoma
- Pilocytic astrocytoma
- Meningioma
- Schwannoma
- DNET
ANSWER:3. Meningioma - F - Cystic or necrotic change may be present - most often in parasagittal tumours (3 – 14%)
- Patient with brain tumor that appears cystic with a solid component— least likely (TW)
- Haemangioblastoma - T - 60% are cystic masses with mural nodule that usually abuts pial surface
- JPA - T - Cerebeallar JPA 30% of total of JPA - Well-circumscribed mass with large cyst, and small reddish-tan mural nodule
- Meningioma - F - Cystic or necrotic change may be present - most often in parasagittal tumours (3 – 14%)
- Schwannoma - T - cystic change is common especially in intraparenchymal schwannomas.
- DNET - T - Well-defined “pseudocystic” lesion (high water content)
- 23.02.24 What is the most common cause of brain abscess in adult?
- Streptococcus & Staph
- Staph & TB
- Staph & Toxo
- TB & Nocardia
- PML
- Streptococcus & Staph
- 65 year old, 3rd yearly follow up scan for CJD. Which is most correct?
- This is expected as CJD is slowly progressive
- Incorrect diagnosis
- Patient most likely has variant CJD
- CJD has variable progression with 10-15% having a long term survival of > 10 years
- Patient more likely to have the more indolent familial form
ANSWER:2. Incorrect diagnosis - T
- 65 year old, 3rd yearly follow up scan for CJD. Which is most correct? (TW)
- This is expected as CJD is slowly progressive - F - rapidly progressive mental deterioration and myoclonus (sCJD). Mean duration of illness for sCJD 4-5months (howevers urvival range varies depending on subtype of sCJD).
- Incorrect diagnosis - T
- Patient most likely has variant CJD - F - mean duration of illness 14months.
- CJD has variable progression with 10-15% having a long term survival of > 10 years - F - both vCJD and sCJD are progressive and uniformly fatal. Occasional case reports of survival to 40-50months, but rare.
- Patient more likely to have the more indolent familial form - F - fCJD accounts for 10-15% cases. Longer survival - mean duration 26 months. Age little younger than sCJD (~60yo). So could be this then - SG
- Bowen Disease
- associated with BCC in 5% of cases if left untreated
- may have a penile cutaneous horn
- results from chronic irritation and/or inflammation with development of scaly palques often involving the meatus
- carcinoma in situ occurring within follicle-bearing epithelium
ANSWER: 4. carcinoma in situ occurring within follicle-bearing epithelium – T - Usually appears as a solitary, dull-red plaque with areas of crusting and oozing.
- Bowen Disease (TW)
- associated with BCC in 5% of cases if left untreated - F - Bowen’s disease - if left untreated - SCC develops in approx 5% of cases.
- may have a penile cutaneous horn - F - penile cutaneous horn is an exophytic keratotic lesion fromed by overgrowth and cornification of the epithelium. CHs typically form on the surface of preexisting lesions such as nevi, warts, or traumatic abrasions.
- results from chronic irritation and/or inflammation with development of scaly plaques often involving the meatus - F - this is leukoplakia.
- carcinoma in situ occurring within follicle-bearing epithelium – T - Usually appears as a solitary, dull-red plaque with areas of crusting and oozing.
- 23.02.74 CNS features NOT typical of TB are ?
- basal meningeal enhancement
- 4cm focal mass
- irregularity of vessels
all are feature of TB haha
ADB–>Original question from G drive
16. 23.02.74 CNS features NOT typical of TB are ?
1. basal meningeal enhancement
2. 4cm focal mass
3. irregularity of vessels
4. hydrocephalus
5. degeneration of central portion/body of corpus callous (false)
- Least likely sites for Toxo in the brain
- GW junction
- Globus Pallidus
- Cerebellum
- Spinal cord
- Putamen
Answer: Spinal cord
STATDX: common locations
- basal ganglia
- Thalami
- corticomedullary junctions
- cerebellum
- MS –least common site
- cerebellar white matter
- cerebellar vermis
- corpus callosum
- Perivenular
NSWER: 2. Cerebellar vermis - F
- Multiple sclerosis, least common site (TW)A
- Cerebellar white matter - T - cerebellar peduncles are classic.
- Cerebellar vermis - F
- Corpus callosum - T - typically mutliple perpendicular callososeptal T2 hyperintensities.
- Perivenular - T - perpendicular lesions in perivenular distribution. >85% lesions periventricular / perivenous. 50-90% callososeptal interface. Perivenular extnsion “Dawson finger” along path of deep medullary veins.
- 23.03.07 In HSV I encephalitis which is TRUE
- Age 50-70 years
- Typically involves superior frontal lobes
- Commonest presentation is headache
- A subacute presentation with ataxia and seizures is recognized
- Oncocytic intracytoplasmic inclusions
**RY - correct answer is actually 4. a subacute presentation is recognized in a subset of individuals. Clinical manifestations include weakness, lethargy, ataxia, seizures - directly from Big Robbins pg 1276 (prior favoured answer was 3)
- 23.03.07 In HSV I encephalitis which is TRUE Rob p828-829
- Age 50-70 years (most common in children and young adults)
- Typically involves superior frontal lobes (predilection for the limbic system (temporal lobes, cingulate gyri, subfrontal region))
- Commonest presentation is headache (alterations in mood,memory and behavior is most common presentation)
- A subacute presentation with ataxia and seizures is recognized
- Oncocytic intracytoplasmic inclusions (Cowdry Intranuclear inclusion bodies)
SCS: two types. Cowdry A: HSV, B: CMV
- Most at risk for cerebral venous infarction
- diabetic in renal failure
- post obstetric patient
- young girl on progesterone only pill
- young girl with SLE
- post obstetric patient - T - think this is most correct.
**LJS - 30-40% SLE pt have antiphospholipid Ab = recurrent arterial and venous thrombosis. ?higher risk than post-partum
***LW: 2019 consensus statement states 5-17% of cerebral venous infarction / thrombosis have SLE related conditions, while 1% to 59% are peri partum and 54% to 71% of cases are on OCP. So i still favour post obstetric patient as most correct option.
(https://www.ahajournals.org/doi/10.1161/STROKEAHA.119.025334)
- Most at risk for cerebral venous infarction (TW) ANSWER: 2
- diabetic in renal failure
- post obstetric patient - T - think this is most correct.
- young girl on progesterone only pill - F - oestrogen is major prothrombotic component. Progesterone much lower risk. Most frequent risk factor for cerebral venous sinus thrombosis is the OCP (but this is presumably the oestrogen containing one).
- young girl with SLE
- Patient with drug resistant Parkinson’s Disease with involvement of autonomic nervous system, caudate, putamen
- Striao-nigral degeneration
- Shy Drager (Now called MSA-C or MSA-P)
- Huntingtons
- Drug resistant Parkinson’s
*AJL - Agree with below. This is now called MSA-P (MSA with parkinsonian features). The other type of MSA is MSA-C (MSA with cerebellar symptoms/changes).
- Shy Drager - T - known as multiple system atrophy. Sky-Drager syndrome still used sometimes for MSA when primary symptoms are autonomic failure. MSA - striatonigral degeneration is clinically similar to idiopathic PD in presentation, but is relatively resistent to L-dopa treatment (due to pattern of neuronal degeneration - ie both dopaminergic projections and its target neurons are absent, therefore L-dopa cannot bulster neurotransmission as occurs in parkinsons disease).
- 23.02.22 Creutz-Jakob disease –what would be UNUSUAL finding ?
- Cortical atrophy is rarely found
- Survival of 3-3½ years
- 23.02.22 Creutz-Jakob disease –what would be UNUSUAL finding ? Rob p830
Answer:2. Survival of 3-3½ years (uniformly fatal usually within 7 months) - Cortical atrophy is rarely found (brain appears grossly normal (no significant atrophy), as progression of dementia so rapid)
- Survival of 3-3½ years (uniformly fatal usually within 7 months)
- Solitary 3cm cystic lesion in the brain containing an opaque gel-like substance
- Hydatid / echinococcus
- Cysticercosis
- Amoebiasis
- Strongyloides
- Ascaris
ANSWER: 11. Hydatid / echinococcus - T - large uni- or multilocular cyst +/- detached germinal membraine. Cyst fluid is opalescent.
- Solitary 3cm cystic lesion in the brain containing an opaque gel-like substance (TW) ANSWER:
- Hydatid / echinococcus - T - large uni- or multilocular cyst +/- detached germinal membraine. Cyst fluid is opalescent.
- Cysticercosis - F - although most common parasitic infection world wide, cyst size is variable and typically 1cm, with range of 4-20mm. Parenchymal cysts 1cm or less, however subarachnoid systs may be larger, up to 9cm reported (and recemose). Cysts are ovoid and white-to-opalescnet, rarely exceeding 1.5cm. Contain an invaginated scolex with hooklets that are bathed in clear cyst fluid.
- Amoebiasis - F - cerebral amebiasis is a rare cause of brain abscess. Amebic encephalitits - hyperintense lesions +/- haemorrhage.
- Strongyloides - F
- Ascaris - F
- HIV positive patient with CD4 count less than 100 has a mass on CT brain. What is the likely diagnosis?
a. Lymphoma
b. Toxoplasmosis
c. Cryptosporidium
d. TB
e. PML
ANSWER:b. Toxoplasmosis - T- most common cause of focal lesion in HIV+.
- HIV positive patient with CD4 count less than 100 has a mass on CT brain. What is the likely diagnosis? (TW)
a. Lymphoma - F - less likely, although increased risk with immunosuppression.
b. Toxoplasmosis - T- most common cause of focal lesion in HIV+.
c. Cryptosporidium - F - less likely cf toxoplasmosis
d. TB - F - less likely
e. PML - F - diffuse/patchy WM abnormalities.
- 23.02.36 Microscopic features of GBM DO NOT include ?
- Necrosis and areas of different histology
incomplete questionRobins: 1308 The histologic appearance of glioblastoma is similar to anaplastic astrocytoma with the additional features of necrosis and vascular/endothelial cell proliferation.Necrosis in glioblastoma often occurs in a serpentine pattern in areas of hypercellularity.Tumor cells collect along the edges of the necrotic regions, producing a histologic pattern referred to as pseudo-palisadingThe vascular cell prolif- eration produces tufts of cells that pile up and bulge into thelumen; the minimal criterion for this feature is a double layer of endothelial cells. With marked vascular cell proliferation the tuft forms a ball-like structure, the glomeruloid body. VEGF, pro- duced by malignant astrocytes in response to hypoxia, contrib- utes to this distinctive vascular change. Since histologic features can be extremely variable from one region to another, small biopsy specimens may not be representative of the entire tumor.
- NF1 – which is not/least associated
- Wilms
- Rhabdomyosarcoma
- CML
- phaeochromocytoma
- osteosarcoma
Answer: Osteosarcoma
RADIOPAEDIA: Assoicated neoplasms
- Pheochromocytoma
- Wilms tumour
- Renal agniomyolipoma
- Rhadbomyosarcoma
- Leiomyosarcoma
- Glimoa: JPA, optic nerve, diffuse brainstem, spinal astrocytoma
- ganglioglioma
- MPNST
- Carcinoid
- Leukaemia
2) ependymoma in the pediatric patient
1. supratentorial location
2. homogenous enhancement
3. hemorrhage is common
4. 15% malignant 5. arise from lumbar nerve roots in the spine
- 15% malignant - ?T - in the literature the percentage of the anaplastic variant ranges from 7-89% (Med and Ped Onc: 1998 30:6 p319).
2) ependymoma in the pediatric patient (TW)
1. supratentorial location - F - infratentorial 70% (floor 4th ventricle). Can also occur supratentorial: frontal > parietal > temporoparietal > juxtaventricular.
2. homogenous enhancement - F - variable heterogeneous enhancement.
3. hemorrhage is common - F - intratumoral haemorrhage 10%
4. 15% malignant - ?T - in the literature the percentage of the anaplastic variant ranges from 7-89% (Med and Ped Onc: 1998 30:6 p319).
5. arise from lumbar nerve roots in the spine - F - arise form ependymal cells lining the central canal.
- 84.APRIL02 Necrosis is a characteristic feature of
- Pilocytic astrocytoma
- Glioblastoma multiforme
- Acoustic schwannoma
- Craniopharyngioma
- Ependymoma
Answer:2. Glioblastoma multiforme
Usually large, heterogenous masses with central necrosis, thick, irregular “shaggy” walls, and increased vascularity with haemorrhage
• Marked mass effect & oedema
• Often find varying degrees of anaplasia within the same tumour
∴biopsy can be misleading
- 84.APRIL02 + APRIL2004 Necrosis is a characteristic feature of
- Pilocytic astrocytoma
- Glioblastoma multiforme
- Acoustic schwannoma
- Craniopharyngioma
- Ependymoma
- Cavernous angiomas, which is not typical:
- Detectable at angiography
- Bleeding tendency
- No intervening normal brain
- Associated venous angioma
- Pseudocapsule
- Detectable at angiography F - most are angiographically occult due to slow flow.
- Cavernous angiomas, which is not typical: (GC)
- Detectable at angiography F - most are angiographically occult due to slow flow.
- Bleeding tendency T - nearly all show evidence of recent and remote hemorrhage, as suggested by the presence of hemosiderin-laden macrophages, cholesterol crystals, and hemosiderin-stained parenchymal tissues.
- No intervening normal brain T - this is the only histopathologic characteristic that distinguishes these lesions from capillary telangiectasias.
- Associated venous angioma T - persistent elevations in venous pressures, as may be seen locally within a venous malformation, may promote a pathologic reactive angiogenesis or angiogenic proliferation, resulting in abnormal blood vessel growth and coalescence. Note that it is the immaturity of the blood vessels within the cavernoma that differentiates it from a venous angioma (which consists of mature vessels responsible for normal venous drainage).
- Pseudocapsule T - clots and blood products of various stages of evolution within the lesion, as well as calcification and gliosis surround the cavernoma, creating the appearance of a pseudocapsule. (eMedicine)
- Huntington’s disease, which is true:
- Autosomal recessive
- Affects the putamen and caudate
- Presents in the second decade
- Chorea due to striatal excitation
ANSWER:2. Affects the putamen and caudate T - degeneration of the striatum (C&P).
- Huntington’s disease, which is true: (GC)
- Autosomal recessive F - autosomal dominant disorder
- Affects the putamen and caudate T - degeneration of the striatum (C&P).
- Presents in the second decade F - age of onset most commonly in 4th and 5th decades, related to the length of the CAG repeat.
- Chorea due to striatal excitation F - loss of striatal inhibitory output, from the degeneration of GABA-containing neurons. This leads to disregulation of the basal ganglia that normally modulate motor output.
- In alcoholic with hyponatremia and partial paresis, which is most likely
- Central pontine myelinosis
- Pontine myelinosis, midbrain and supratentorial changes
- Pontine lesion secondary to Thiamine deficiency
- Subacute combined degeneration of cord secondary to thiamine deficiency
- Cord degeneration secondary to folate deficiency
- Hashimoto’s thyroiditis
Answer: Pontine myelinosis, midbrain, and supratentorial changes aka “Osmotic demyelination syndrome.
Osmotic Demyelination syndrome
- recent term replacing CPM, recognising that extrapontine structures can also be affected. j
- includes CPM and extrapontine myelinolysis.
- has a biphasic pattern.
- 1s phase is usually attributable to the electrolyte abnormality. 2nd phase follows rapid reversal of the abnormality progressing to classic osmotic demyelination syndrome.
- when pontine involvement is prominent, clinical features consist of:
–> spastic quadriparesis
–> pseudo bulbar palsy
–> changes in levels of consciousness
–> coma
–> death
THIAMINE DEFICIENCY
- causes Wernicke encephalopathy.
- characterised by combination of psychotic symptoms and ophthalmoplegia.
- characterised by foci of haemorrhage and necrosis in the maxillary bodies and the walls of the 3rd and 4th ventricles.
VITAMINE B12 Deficiency
- Causes subacute combined degeneration of the spinal cord
- affects both ascending and descending spinal tracts.
- presents with bilateral symmetrical numbness, tingling, and slight ataxia int he lower extremities.
- Least likely site for hypertensive bleed in the brain is:
- Hippocampus
- Cerebellum
- Basal ganglia
- Thalamus
- Brainstem
- Hippocampus
- Least likely site for hypertensive bleed in the brain is: (GC)
- Hippocampus T
- Cerebellum F - 10%; behave like SOL’s - herniation & obstructive hydrocephalus.
- Basal ganglia F - with thalamus account for 65% of hypertensive bleeds
- Thalamus F - see above
- Brainstem F - 10%
- Sep03.28 Pilocytic Astrocytoma
- Associated with NF2
- 50% are solid
- Prognosis is less than 70% 5 year survival rate
- Multipolar cells with microcysts, and bipolar cells with Rosenthal fibres
Answer: Multipolar cells with micro cysts and bipolar cells with Rosenthal Fibers
FROM ROBBINS: Pilocytic astroctyomas
- WHO I
- Children/young adults
- located in cerebellum (60%), optic nerves (25-30%), floor/walls 3rd ventricles, cerebral hemispheres.
- rarity of TP53 mutations
- NF1 association
- Large cystic component with enhancing mural nodule (67%)
- Completely solid (17%). Heterogenous mixed cystic/solid (16%)
- Tumors often biphasic, microcytic and fibrillary areas. Contain bipolar cells with GFAP positive processes, Rosenthal fibres, eosinophilic granular bodies.
- 5-10 year survival rate >95%
- Subependymoma
- Usually slow growing and asymptomatic
- Associated with TS
- 5-10 yo
- Commonly in lateral ventricle
Answer: usually slow growing and asymptomatic
Robbins
Subependymomas are solid, sometimes calcified, slow-growind nodules attached to the ventricular lining and protrued into the ventricle.
Usually asymptomatic, incidental finding on autopsy/imaging.
Most often found in lateral and 4th ventricles.
Radiopaedia
- 4th ventricle (50-60%)
- lateral ventricles, usually frontal horns (30-40%)
- 3rd ventricle (rare)
- central canal (rare).
- The following are true in relation to cerebello-pontine angle tumours:¬
- Type 2 neurofibromatosis is associated with multiple schwannomas
- Acoustic neuromas are typically hyperintense on TI weighted images
- Peripheral enhancement is a feature of epidermoid cysts
- Choroid plexus seen in the cerebello-pontine angle is not a normal feature
- Acoustic neuromas arise from the cochlear portion of the acoustic nerve
- Type 2 neurofibromatosis is associated with multiple schwannomas – T – MISME, multiple inherited schwannomas, meningiomas, Ependymoma.
- The following are true in relation to cerebello-pontine angle tumours:¬ (TW)
- Type 2 neurofibromatosis is associated with multiple schwannomas – T – MISME, multiple inherited schwannomas, meningiomas, Ependymoma.
- Acoustic neuromas are typically hyperintense on TI weighted images – F – intermediate signal most common. High signal patches if rare haemorrhagic lesion present.
- Peripheral enhancement is a feature of epidermoid cysts – F – no enhancement is the rule, however sometimes the margin of cyst minimally enhances CECT. Mild peripheral enhancement occurs in approx 25% cases. Osborn.
- Choroid plexus seen in the cerebello-pontine angle is not a normal feature – F – choroid plexus may normally pass from 4th ventricle through foramen of Luschka in to CPA cistern. Osborn.
- Acoustic neuromas arise from the cochlear portion of the acoustic nerve – F – arise from the vestibular segment of CN VIII, with an equal frequency b/w superior and inferior vestibular nerves. Cochlear nerve involvement is much less common (15% vs 85%).
- 23.02.42 Commonest sites for ependymoma are ?
- Fourth ventricle in children and spine in adults
- Lateral and third ventricle in infants…..
- Periventricular areas (permutations of adult/infant/children)
- Fourth ventricle in children and spine in adults
- White matter disease least likely
- Picks
- ADEM
- DAI
ANSWER: 11. Pick’s disease - focal cortical atrophy of anterior frontal and anterior temporal lobes
- White matter disease, least likely: (GC)
ANSWER: 11. Pick’s disease - focal cortical atrophy of anterior frontal and anterior temporal lobes. - ADEM
- DAI
- 23.02.34 Ganglioglioma ? 1. Superficial location
(Most commonly superficial and supratentorial (temporal > frontal > parietal))
- Children with Ependymoma, which is false:
- Prognosis poor – cannot remove completely
- Less common than medulloblastoma
- Does not metastasize as frequently as medulloblastoma
- Myxopapillary variant in filum terminale
- 4 year survival with 50%
- 4 year survival 50% F - current 5YS rate for patients with intracranial ependymomas is approximately 50%, when rates from children and adults are combined. Stratification based on age reveals 5YS rates of 76% in adults and only 14% in children.
- Children with Ependymoma, which is false: (GC)
- Prognosis poor – cannot remove completely T - Despite the survival advantage of gross total resection, lesions of the posterior fossa are in close proximity to cranial nerves with a significant risk of long-term neurologic dysfunction and disability. Hence, most tumors of the posterior fossa cannot be fully resected and are likely to recur without postoperative radiation.
- Less common than medulloblastoma T - third most common paediatric brain tumour, after medulloblastoma & JPA.
- Does not metastasize as frequently as medulloblastoma T - subarachnoid dissemination via CSF rare, observed in <10% of patients at diagnosis when ependymoblastomas are excluded. The incidence is higher with infratentorial ependymomas than with supratentorial tumors (9% vs 1.6%).
- Myxopapillary variant in filum terminale T - considered a biologically and morphologically distinct variant of ependymoma, occurring almost exclusively in the region of the cauda equina and behaving in a more benign fashion than grade II ependymoma.
- 4 year survival 50% F - current 5YS rate for patients with intracranial ependymomas is approximately 50%, when rates from children and adults are combined. Stratification based on age reveals 5YS rates of 76% in adults and only 14% in children.
- Meningioma, which is least correct:
- WHO class 1
- Intra axial position
- Invasion into brain parenchyma does not change grade
- Papillary variant
Answer: Intra-axial position (least correct).
ROBBINS
- Typical meningiomas are WHO I
- The presence of brain invasion is associated with increase risk of recurrence, but does not alter the histologic grade of the lesion.
Histologic Variants
- WHO I: Syncytial, fibroplastic, transitional, psammomatous, secretory, microcystic
- WHO II: clear cell, chordoid.
- WHO III: papillary, rhabdoid.
- 15yo with psychosis, MRI showed diffuse WM abnormality, which is most likely:
- Metachromic leukodystrophy
- ADEM
- Huntington’s
- HIV
- Metachromic leukodystrophy T - progressive symmetrical areas of T2 hyperintensity, (confluent “butterfly-shaped” PVWM change), early sparing of the subcortical U-fibres. Late onset form (after puberty-4th decade) may manifest as pychiatric/behavioural OR CNS motor signs + peripheral neuropathy. Other forms: late infantile (6m-3yo, death in 5yrs), juvenile (4-12yo, slower progressn).
- 15yo with psychosis, MRI showed diffuse WM abnormality, which is most likely: (GC)
- Metachromic leukodystrophy T - progressive symmetrical areas of T2 hyperintensity, (confluent “butterfly-shaped” PVWM change), early sparing of the subcortical U-fibres. Late onset form (after puberty-4th decade) may manifest as pychiatric/behavioural OR CNS motor signs + peripheral neuropathy. Other forms: late infantile (6m-3yo, death in 5yrs), juvenile (4-12yo, slower progressn).
- ADEM F - multifocal punctate or large confluent areas of T2/FLAIR hyperintensity.
- Huntington’s F - caudate nucleus atrophy.
- HIV F - AIDS-dementia complex: cerebral atrophy, subtly increased SI on T2/FLAIR without mass effect (from leaky capillaries), may be focal/diffuse, symmetric/asymmetric, reversible/nonreversible. Symptoms are more of inattention, indifference and psychomotor slowing (progressing to frank dementia), i.e. not psychosis.
- 23.02.25 Alcoholic, treatment for Wernickes has rapid change in heart size over 1 wk. Most likely:
- Dehydration
- Resolution of pericardial effusion
- Projectional change on CXR
- Beri-beri heart disease
- Beri-beri heart disease this is caused by vit B1 (Thiamine) deficiency can cause acute heart failure with decreased LV systolic function and enlargement +/-
- 23.03.10 In a alcoholic with hyponatraemic and parietal paresis, which is MOST LIKELY:
- Central pontine myelinolysis
- Pontine myelinolysis, midbrain and supratentorial diseases
- Pontine lesions secondary to thiamine defiency
- Subacute combined degeneration of cord secondary to thiamine defiency
- Cord degeneration secondary secondary to folate defiency
*LW:
Correct terminology is osmotic demyelination syndrome / osmotic myelinolysis; which is a global term of both central pontine and extra pontine myelinolysis.
Central pontine myelinolysis clinical features:
dysarthria and dysphagia (secondary to corticobulbar fibre involvement), a flaccid quadriparesis (from corticospinal tract involvement) which later becomes spastic, all from involvement of the basis pontis.
if the lesion extends into the tegmentum of the pons - pupillary, oculomotor abnormalities may occur. There may be an apparent change in conscious level reflecting the “locked-in syndrome” that a large lesion in this site is particularly liable to produce.
Extra pontine myelinolysis clinical features:
Movement disorders
Mutism, parkinsonism, dystonia, and catatonia
- Central pontine myelinolysis
- Huntington’s chorea, what does it show:
- Various locations including basal ganglia
- Caudate nucleus + putamen
- Cerebellum
- Locus cereus
- Caudate nucleus + putamen T - striking atrophy (heart or box-shaped frontal horns), less dramatic change in the putamen.
- Huntington’s chorea, what does it show: (GC)
- Various locations including basal ganglia F - although the globus pallidus may secondarily atrophy, with dilatation of the lateral and third ventricles.
- Caudate nucleus + putamen T - striking atrophy (heart or box-shaped frontal horns), less dramatic change in the putamen.
- Cerebellum F
- Locus ceruleus F - involved in Parkinson’s disease.
- 23.03.87 Patient with drug resistant Parkinsons and autonomic neuropathy ?
- Shy Drager Syndrome
- Drug resistant Parkinsons
- Striatnigeral degeneration
- Olviopontocerebellar atrphy
- Progressive supranuclear palsy
- Huntingtons
- Shy Drager Syndrome
SCS: Radiopaedia. Option 1, 3 and 4 all come under the umbrella of multisystem atropy (MSA), but are old terms superceded by MSA-C and -S
- Shy Drager synd: when autonomic symptoms predominate (now abandoned)
-Striatonigral degeneration: shows predominant Parkinsons features (Now known as MSA-P)
- Olivopontocerebellar atrophy: cerebellar dysfunction dominates (now known as MSA-C = hot cross bun sign
• Parkinsonism with autonomic
system failure, often manifesting as orthostatic hypotension
o orthostatic hypotension
o urinary incontinence
o inability to sweat
• Loss of neurones from intermediolateral column of spinal cord
o explains sympathetic dysfunction
• May be similar pathologically to Parkinson disease (Lewy bodies) or striatonigral degeneration (widespread neuronal loss)
• Shy Drager Syndrome
• Multiple system atrophy with autonomic failure; is a progressive disorder of the central and autonomic nervous systems. The disorder is characterized by postural hypotension
• There are 3 types of Shy-Drager syndrome:
o Parkinsonian-type which may include symptoms of Parkinson’s disease such as slow movement, stiff muscles, and mild tremors
o cerebellar-type which may include problems such as loss of balance and the tendency to fall; and
o combination-type
- Pilocytic Astrocytoma, most common location:
- Cerebrum
- CPA
- Brainstem
- Hypothalamus
- Pituitary
Answer: Hypothalamus
Robbins
- Usually located in the cerebellum, but may also appear in the floor and walls of the 3rd ventricle, optic nerves, and occasionally the cerebral hemispheres
Statdx
- NF1 individuals: Optic pathway
- others: Cerebellum (60%) > hypothalamus, brainstem, cerebral hemispheres > optic pathway
Radiopaedia
- Cerebellum (60%)
- Optic pathway (optic nerve, optic chiasm, hypothalamus, optic radiation), 25-30% particularly common in NF1
- Other less common locations including brainstem, cerebral hemispheres, cerebral ventricles
Schwannoma versus plexiform neurofibroma
SCHWANNOMA
- well-circumscribed, encapsulated masses, that abut the associated nerve without invading it.
- comprised of an admixture of dense and loose areas referred to as ANTONI A and ANTONI B areas, respectively.
- palisading of nuclei is common and “nuclear-free zones” that lie between regions of nuclear palisading are termed VEROCAY BODIES.
- Within the cranial vault: most common location is CP angle. Other locations include trigeminal nerve and dorsal roots.
- Extradural: large nerve trunks or as soft tissue lesions.
- no risk for malignant transformation.
- epidemiology: 5-6th decade. NF2.
PLEXIFORM NEUROFIBROMA
- neurofibromas are neoplastic Schwann cells admixed with perineurial-like cells, fibroblasts, mast cells, and CD34+ cells.
- plexiform neurofibromas specifically: uncommon variant of neurofibromas. They grow within and expand nerve fascicles, entrapping associated axons - “bag of worm” appearance.
- Unlike other neurofibroma types, plexiform neurofibromas are at significant risk of eventual malignant transformation (5-10%).
- epidemiology: early childhood. sporadic or NF1-associated.
- 23.03.12 Child with post paravertebral mass, biopsy shows acute neural elements and schwann cells not attached to nerve – DIAGNOSIS ?
- Ganglioneuroma
- Ganglioglioma
- Neurofibroma
- Schwannoma
- Neuroblastoma
Answer: Ganglioneuroma
FROM ROBBINS/RADIOPAEDIA/STATDX
GANGLIONEUROMA
- One of the tumors from the “Neuroblastic tumor family” which include ganglioneuroblastoma and neuroblastoma.
- fully differentiated neuronal tumors derived from primordial neural crest cells that form the SNS.
- Composed of ganglion and Schwann cells + fibrous tissues.
- Age: 40% <1 year old, 35% 1-2 years old, 25% >2 year old.
- presentation. Incidental finding.
- Associations: MEN type IIB (particularly with mucosal ganglioneuromas.
NEUROBLASTOMA
- most important member of the “Neuroblastic tumor family”
- Classically - composed of small primitive-appearing cells.
- Typically, Rosettes (Homer-Wright pseduorosettes) can be found.
- median age at diagnosis 18 months (40% diagnosed in infancy)
- Mostly sporadic, 1-2% familial.
- 60-80% of children present with stage 3 or stage 4 tumors.
- IN childhood, 40% of neuroblastoma arise in the adrenal medulla. presents as large abdominal mass with fever and weight loss.
- the remainder occur along the sympathetic chain - paravertebral abdomen (25%), posterior mediastinum (15%).
GANGLIOMA
- Neuronal tumor family.
- most common of the neuronal tumors of the CNS
- composed of mixture of mature neuronal and glial cells.
- Typically superficial lesions that present with seizures.
- 20% have BRAF mutation.
NEUROFIBROMA
- Each fascicle is infiltrated by neoplasm – not possible to separate lesion from nerve)
SCHWANNOMA
- most common in 5th to 6th decade except in NF-2)
- 25yo man with liver failure, prev MRI showing increased signal in basal ganglia, most likely:
- HIV
- Wilson’s
- Liver failure kernicterus
- Disseminated toxoplasmosis
- Wilson’s - T - Onset of liver diease with WD usually 8-16yo, neurological symtpoms rare <12yo. WD often recognised in 2nd-3rd decade. M=F. Most common to see putamen (predilection for outer rim) and pons (dorsal and central regions) of increased T2.
- 25 yo man with liver failure, previously had MRI showing increased signal in basal ganglia, most likely: (T bag, G bees)
- HIV - F
- Wilson’s - T - Onset of liver diease with WD usually 8-16yo, neurological symtpoms rare <12yo. WD often recognised in 2nd-3rd decade. M=F. Most common to see putamen (predilection for outer rim) and pons (dorsal and central regions) of increased T2.
- Liver failure kernicterus - F - develops during the 1st year of birth (term and pre-terms). Kernicterus is the staining of the basal ganglia, brainstem, and cerebellum by unconjugated bilirubin: encephalopathy due to deposition of toxic unconjugated bilirubin. High signal T2 in globus pallidus or other areas of basal ganglia. Most common cause = erythroblastosis fetalis.
- Disseminated toxoplasmosis - F
- 23.03.90 Periventricular mass in patient with renal transplant
- Lymphoma - primary
- GBM
- Lymphoma – 2°
Answer: Lymphoma - primary
FROM ROBBINS
Primary CNS lymphoma is the most common CNS neoplasm in immunosuppressed individuals, including those with AID and immunosuppression after transplantation.
Lymphoma arising outside the CNS rarely involves the brain parenchyma.
FROM STATDX
Post-transplant lymphoproliferative disease (PTLD) is a spectrum of abnormal lymphoproliferations resulting from trasnplantations
Ranges from lymphoid hyperplasia to malignant lymphoma.
Most common B-lymphocyte proliferation is associated with EBV infection.
It is the most common malignancy in kidney transplant recipients after skin cancer.
Renal transplant patients have a 35x increased risk of developing lymphoma.
Most common site is the kidney graft.
Retroperitoneum is the most common site of lymphadenopathy.
Other sites include GI tract, liver, spleen, lung, head & neck - involvement in periventricular regions and subcortical white matter.
- Craniopharyngioma on histology in a 60 yo, solid 5cm, no cystic change or calcification:
a. Review pathology: not usually solid tumours
b. Metastatic carcinoma is excluded
c. Papillary variant
d. Absence of calcification is unusual
c. Papillary variant
- Craniopharyngioma on histology in a 60 yo, solid 5cm, no cystic change or calcification: (GC)
a. Review pathology: not usually solid tumours F - vary from solid to cystic.
b. Sellar expansion but not destruction F - extensive sellar destruction in 75% of craniopharyngiomas
c. Papillary variant T
d. Absence of calcification is unusual F - only 30-40% contain Ca2+ in adults, cf. 90% of CP in children.
Craniopharyngiomas arise from epithelial remnants of Rathke’s pouch that are trapped in pituitary stalk. Grows slowly and damages hypothalamus (causing endocrine abnormalities), compresses optic chiasm (causing bitemporal hemianopsia), blocks third ventricle (causing hydrocephalus).
Histologically benign but frequently recurs due to incomplete excision; rarely metastasizes or transforms to squamous cell carcinoma.
* Papillary subtype: usually occurs in older adults (50+). Histology: well-circumscribed, composed of cores of fibrovascular stroma lined by well-differentiated squamous epithelium that may separate to form pseudopapillae; resembles squamous papilloma; no stellate reticulum, no “wet” keratin, no calcification, no xanthogranulomatous inflammation; cystic fluid does not resemble motor oil.
* Adamantinomatous subtype: usually children (5-14yrs), associated with beta-catenin mutations. 90% cystic, 90% calcifn. Histol: wet keratin, Ca2+, cysts with cholesterol (sump oil).[Path outlines]
- Which of the following is least correct regarding fat embolism?
a. Symptoms of irritability and confusion
b. Petechial rash
c. Hypovolaemic shock
d. Passive flow of adipose tissue through the vascular system
Answer: Hypovolemic shock (least correct)
Radiopaedia/Robbins
- incidence of 1-3% following long bone fractures and 33% following bilateral long bone fractures
- Sx: variable, headache, lethargy, irritability, deliuim, stupor, convulsions, coma
- Can reach brain through right-left cardiac shunt or through intact pulmonary circulation.
- A diffuse petechial rash (20-50% of cases) is related to rapid onset of thrombocytopenia.
- 23.03.84 Cystic tumour in brain LEAST LIKELY:
- Haemangioblastoma
- JPA
- PNET
- Meningioma
- Schwannoma
- DNET
Answer:4. Meningioma (Cystic or necrotic change may be present - most often in parasagittal tumours (3 – 14%))
- 23.03.84 Cystic tumour in brain LEAST LIKELY is ?
- Haemangioblastoma (60% are cystic masses with mural nodule that usually abuts pial surface)
- JPA (Cerebeallar JPA 30% of total of JPA - Well-circumscribed mass with large cyst, and small reddish-tan mural nodule)
- PNET (commonly cystic)
- Meningioma (Cystic or necrotic change may be present - most often in parasagittal tumours (3 – 14%))
- Schwannoma (cystic change is common)
- DNET (Well-defined “pseudocystic” lesion (high water content)) BR 1348 – cystic changes are a common finding
- Peripheral MCA aneurysm, most likely history:
- SLE
- Past history of irradiation for fibrous dysplasia
- Hypertension
- History of rheumatic fever and tooth extraction
- History of rheumatic fever and tooth extraction T - Aneurysms that develop at distal sites in the intracranial circulation are often caused by trauma or infection. Mycotic aneurysms are most commonly due to infected arterial embolus, occurring in subacute bacterial endocarditis. In such cases, they are often multiple, being situated in small corticomeningeal branches of the cerebral artery.
- Peripheral MCA aneurysm, most likely history: (GC)
- SLE F - Commonly reported CNS vascular lesions with SLE include infarcts and transient ischemic attacks. Intracranial hemorrhages are present in approximately 10% of patients with CNS symptoms. Although uncommon, arteritic and nonvasculitic aneurysms occur in SLE. These can be saccular, fusiform, or a bizarre-looking mixture of both.
- Past history of irradiation for fibrous dysplasia F - Radiation is contraindicated as it has been found to increase the rate of secondary malignancy by 400 times. If in the remote past… intracranial aneurysms may develop (and rupture) post irradiation.
- Hypertension F - Charcot-Bouchard microaneurysms secondary to segmental lipohyalinosis of the walls of long penetrating arteries.
- History of rheumatic fever and tooth extraction T - Aneurysms that develop at distal sites in the intracranial circulation are often caused by trauma or infection. Mycotic aneurysms are most commonly due to infected arterial embolus, occurring in subacute bacterial endocarditis. In such cases, they are often multiple, being situated in small corticomeningeal branches of the cerebral artery.
3) Sellar lesions, which is true
- normal sella favours meningioma rather than macroadenoma
- squamous variant of craniopharyngioma is more likely cystic
- intrasellar arachnoid cyst will displace stalk anteriorly
- 17mm pituitary is normal for pregnant female
- macroadenoma more likely to compress ICA than meningioma
normal sella favours meningioma rather than macroadenoma - T - as pituitary MAs grow they first expand sella and then grow upwards. Aggresive adenomas extend inveriorly, invade sphenoid, and may destroy upper clivus. As they are soft - often are indented by diaphragma sellae giving them a ‘snowman’ configuration which can help Dx from meningoma. )
Sellar lesions, which is true (TW)
- normal sella favours meningioma rather than macroadenoma - T - as pituitary MAs grow they first expand sella and then grow upwards. Aggresive adenomas extend inveriorly, invade sphenoid, and may destroy upper clivus. As they are soft - often are indented by diaphragma sellae giving them a ‘snowman’ configuration which can help Dx from meningoma.
- squamous variant of craniopharyngioma is more likely cystic - F - squamous papillary type (more common in adults) rarely calcifies, often solid, isodense. Both adamantinomatous and squamous papillary types enhance.
- intrasellar arachnoid cyst will displace stalk anteriorly - F - 10% arachnoid cysts are suprasellar & would push stalk back.
- 17mm pituitary is normal for pregnant female - F - normal pituitary measurements: children 6mm, males and post-menopausal females 8mm, young menstruating females 10mm, pregnant/lactating females 12mm.
- macroadenoma more likely to compress ICA than meningioma - F- meningioma more likely to compress / constrict ICA (given ICA course in cavernous sinus too). Note that macroadenomas can also extend into the cavernous sinus.
- 85.APRIL02 A 12-year-old boy presents with a seizure and a solid mass involving the cortex of a temporal lobe is demonstrated on MRI. The most likely diagnosis is:
- DNET
- Pilocytic astrocytoma
- Anaplastic astrocytoma
- Mesial temporal sclerosis
- Pleomorphic xantho-astrocytoma
*LW: Difficult question, possibly poor recall given no perfect answer:
Preferred options of DNET vs PXA
1) DNET: multicystic bubbly cortical mass, commonly temporal lobe
2) Pilocytic astrocytoma: enhancing nodule with associated cyst, rarely cortical.
3) Anaplastic Astrocytoma may cause solid appearing cortical mass, however anaplastic occur in adulthood mean age - 40-50yrs.
4) Mesial sclerosis: neronal loss and gliosis
5) Pleomorphic xanthoastrocytoma: cortical enhancing mass with adjacent cyst, temporal lobe most common, older children.
Previous notes:
i thin it is 3 or 5: in the absence of stated associated cystic component astrocytoma possible, while age argues against. However question states age and temporal lobe, thus PXA felt most likely.
- Brain abscess, which are most likely organisms:
a. Gram negative anaerobes
b. Streptococci and staphylococci
c. Haemophilus and menigococcus
d. Haemophilus and pneumococcus
e. Staphylococci and mycobacteria
Streptococci and staphylococci
Nearly always caused by bacterial infections; direct implantation / local extension / haematogenous spread. 25% are cryptogenic.
Predisposition:
- diabetes
- immunosuppressed
- acute bacterial endocarditis (usu multiple),
-cyanotic CHD (R-L shunt), chronic pulm sepsis.
CSF: elevated white cell count and protein, normal glucose.
Complications:
- rupture with ventriculitis/meningitis
- daughter abscesses
- venous sinus thrombosis & infarction,
- mass effect. [Robbins]
- Cavernous angiomas, which is not typical:
- Detectable at angiography
- Bleeding tendency
- No intervening normal brain
- Associated venous angioma
- Pseudocapsule
- Detectable at angiography F - most are angiographically occult due to slow flow.
- Child with posterior paravertebral mass, biopsy shows mature neural elements and Schwann cells not attached to nerve – diagnosis is
- Ganglioneuroma
- Ganglioglioma
- Neurofibroma
- Schwannoma
- Neuroblastoma
Answer: Ganglioneuroma
NEUROBLASTIC TUMORS
Includes tumors of the sympathetic ganglia and adrenal medulla that are derived from primordial neural crest cells populating these sites. Includes Neuroblastoma, ganglioneuroblastoma, and glanglioneuroma.
Neuroblastoma
- most important member of the family.
- most common extra cranial solid tumor of childhood
- 40% arise in the adrenal medulla. 25% paravertebral abdomen, 15% posterior mediastinum.
- histology: undifferentiated high grade. small primitive cells with dark nuclei. Homer-wright pseudoroettes.
Ganglioneuroblastoma
- commonly attached to a nerve trunk or arise form the adrenal gland.
- contains elements of both neuroblastoma and glanglioneuroma
Ganglioneuroma
- fully differentiated neuronal tumour without immature elements.
- occur anywhere along the peripheral autonomic ganglion sites.
- posterior paraspinal mediastinum is most commonest location (40%).
- other locations include retroperitoneum, neck, and adrenal glands.
- histology: neoplastic ganglion cells. Spindle-shaped Schwann cells are present in the background storm.
PERIPHERAL NERVE SHEATH TUMORS (PNET)
Benign and malignant neoplasms, majority composed of cells that show evidence of Schwann cell differentiation. These include: Schwannoma, neurofibroma, and malignant peripheral nerve sheath tumor (MPNST).
Schwannoma
- A component of NF2 (chromosome 22).
- well circumscribed encapsulated assess that abut the associated nerve without invading it.
- Composed of an admixture dense/loos areas i.e. Antoni A and Antoni B areas.
- Palisading of nuclei is common, regions between are called Verocay Bodies
- Immunohistochemical staining: S100+
- Within cranial vault: most common at CP angle (CNVIII). can affect CNV and dorsal roots)
- Extradural: large nerve trunks or as soft tissue lesions without an identifiable associated nerve.
Neurofibroma
- Benign nerve sheath tumor, more heterogenous than schwannoma
- contains neoplastic Schwann cells admixed with perineurial-like cells, fibroblasts, mast cells, and CD34+ spindle cells
- A component of NF1
- Diffuse neurofibromas: plaque like elevation of skin
- Plexiform neurofibromas: deep or superficial locations in association with nerve roots/large nerves.
Malignant peripheral nerve sheet tumor (MPNST)
- High grade tumors
- half arise in NF1 patients, presumed malignant transformation of plexiform
- “triton tumor” has focal areas with rhabdomyoblastic morphology.
OTHER
Ganglioglioma - F - tumor of neoplastic astrocytes (rarely oligodendrocytes) and ganglion cells.
